Haem - Myeloma

Question 1

What is myeloma?

Answer 1

Cancer affecting plasma cells in the bone marrow

Cancer in a specific plasma cell causes large production of a specific paraprotein , which is an abnormal antibody or part of an antibody

Question 2

What are plasma cells?

Answer 2

B lymphocytes that produce antibodies

Question 3

What is multiple myeloma?

Answer 3

Myeloma affects multiple bone marrow areas in the body

Question 4

What is monoclonal gammopathy of undetermined significance?

Answer 4

Production of a specific paraprotein without other features of myeloma or cancer

Incidental finding in otherwise healthy person

Small risk of progression to myeloma (1% per year)

Question 5

What is smouldering myeloma?

Answer 5

Abnormal plasma cells and paraproteins

No organ damage or symptoms

Greater risk of progression to myeloma (10% per year)

Question 6

What is the pathophysiology of myeloma?

Answer 6

Plasma cells are B lymphocytes that have produce a specific antibody
There are 5 types of antibodies, AGMDE

Myeloma is a cancer of a single plasma cell, with a genetic mutation causing rapidly uncontrollable multiplication

Leads to paraproteinemia due to abnormally high production of paraprotein

Question 7

In myeloma what part of the antibody is affected more commonly?

Answer 7

Light chain

Question 8

What are Bence Jones proteins?

Answer 8

Free light chains in urine

Question 9

What are the features of myeloma?

Answer 9

CRAB

Calcium (raised)
Renal failure
Anaemia
Bone lesions and bone pain

Question 10

What is the most common complication of myeloma?

Answer 10

Pancytopenia

Bone marrow is invaded causing suppression of blood cell lines leading to anaemia, leukopenia and thrombocytopenia

Question 11

What type of anaemia occurs in myeloma?

Answer 11

Normocytic normochromic

Question 12

What causes myeloma bone disease?

Answer 12

Increased osteoclast activity and suppressed osteoblast activity

Caused by cytokine release from abnormal plasma cells and other nearby cells

Patchy bone metabolism, does not affect everywhere

Question 13

What are the common sites of myeloma bone disease?

Answer 13

Skull
Spine
Long bones
Ribs

Question 14

What are the patches of thin bone called in myeloma bone disease?

Answer 14

Osteolytic lesions

Question 15

What does myeloma bone disease lead to?

Answer 15

Pathological fractures
Vertebral body in spine can collapse of femur fracture under minimal force

Hypercalcaemia
Increased osteoclast activity

Plasmacytomas
Individual tumours formed by cancerous plasma cells
Occur in bones replacing normal bone tissue or in soft tissues

Question 16

Why do patients with myeloma often develop renal impairment?

Answer 16

Paraproteins
Deposited in kidneys

Hypercalcaemia

Dehydration

Glomerulonephritis

Medications
Used to treat condition

Question 17

What is the normal plasma viscosity vs water?

Answer 17

1.3-1.7 x

Question 18

When does plasma viscosity increase?

Answer 18

More proteins in blood e.g. paraproteins in myeloma

Question 19

Why is hyperviscosity syndrome an emergency?

Answer 19

Causes many issues
- Bleeding (nosebleeds and gums)
- Visual symptoms and eye changes (retinal haemorrhages)
- Neurological complication (stroke)
- Heart failure

Question 20

What are the risk factors of myeloma?

Answer 20

Older age
Male
Black
Family history
Obesity

Question 21

What are the presenting features of myeloma?

Answer 21

Persistent bone pain (spinal pain)
Pathological fractures
Unexplained fatigue
Unexplained weight loss
Fever of uknown origin
Hypercalaemia
Anaemia
Renal impairment

Question 22

What investigations are used for myeloma?

Answer 22

FBC - anaemia or leukopenia
Calcium - raised
ESR - raised
Plasma viscosity - raised
U&Es - renal impairment
Urine protein electrophoresis - Bence-Jones protein
Serum protein electrophoresis - detect paraproteinaemia
Serum-free light-chain assay - detect abnormally abundant light chains

Question 23

What is required to confirm diagnosis of myeloma?

Answer 23

Bone marrow biopsy required for diagnosis and perform cytogenic testing

Question 24

What imaging is used to assess for bone lesions in myeloma?

Answer 24

Whole body MRI
Whole-body low-dose CT
Skeletal survey

Question 25

What are the typical x-ray changes in myeloma?

Answer 25

Well-defined lytic lesions (punched out)
Diffuse osteopenia
Abnormal fractures

Raindrop skull or pepper pot skull, multiple lytic lesions in skull on xray

Question 26

How is myeloma managed?

Answer 26

Oncology and haematology MDT

Aim is to control disease, never fully cured, relapsing-remitting course

Question 27

How is myeloma treated?

Answer 27

Combination of chemotherapy
- Bortezomib (proteasome inhibitor)
- Thalidomide
- Dexamethasone

High-dose chemotherapy followed by stem cell transplant option for fitter patients, longer remission stem cell transplant can be
- Autologous (own stem cells)
- Allogenic (healthy donor)

Question 28

How is myeloma bone disease managed?

Answer 28

Bisphosphonates - to suppress osteoclast activity
Radiotherapy - bone lesions can improve bone pain
Orthopaedic surgery - stabilise bones or treat fractures
Cement augmentation - injecting cement into fractures or lesions to improve spine stability or pain

Question 29

What are the complications of myeloma?

Answer 29

Infection
Bone pain
Fractures
Renal failure
Anaemia
Hypercalcaemia
Peripheral neuropathy
Spinal cord compression
Hyperviscosity syndrome
VTE